Fibrous Dysplasia

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Introduction

Fibrous dysplasia is an uncommon bone disorder characterized by an abnormal bone growth where normal bone is replaced with fibrous bone tissue. This can cause deformation and fracture of the bones. It is non-neoplastic tumor-like congenital process.

Cause

Fibrous dysplasia is linked to a gene mutation that affects the cells that produce bone. The medullary cavity of bones is filled with fibrous tissue, causing the expansion of the areas of bone involved. The bony trabeculae are abnormally thin and irregular in shape. The cause of this may be linked to transcription factor C-fos. The activity of C-fos increases, leading to over expression of the gene, and hence tumor formation.

Treatment

Usually, no treatment is required as the bone lesions usually do not progress beyond puberty. As for drugs, pain killer and supplements are given. In severe cases surgery is preferred.Fibrous Dysplasia Treatment. Surgery may be necessary for more problematic cases of fibrous dysplasia involving chronic pain, fractures, or deformity.

Statistics

Fibrous dysplasia presents clinically in children and adolescents, with a median onset age of 8 years. The exact incidence and prevalence of the disorder is unknown.In the fibrous dysplasia there is an a!- most 2 : 1 male predominance in 427 patients with the monostotic variant, and 1 .3 : 1 male predominance in 74 patients with the polyostotic form of this disorder.